全长BP180和天然BP180-α6β4整合素复合物ELISA在血清学不确定的黏膜类天疱疮中的诊断效用。

文献信息

中文摘要

黏膜类天疱疮（MMP）是一种慢性自身免疫性黏膜表面上皮下大疱性疾病，可导致不可逆的瘢痕形成、失明或吞咽困难。早期诊断具有挑战性，因为组织病理学无特异性，且常规BP180 NC16A ELISA的敏感性有限。我们在六例疑似口腔或眼部MMP患者中评估了使用全长重组BP180和天然BP180-α6β4整合素复合物的ELISA。常规NC16A ELISA在六例患者中有四例为阴性，且仅凭活检往往不足以诊断。当与直接免疫荧光（DIF）和临床表型结合解读时，这两种检测在一部分病例中提供了有用的血清学支持，而在其他病例中DIF仍是诊断的主要依据。因此，这些检测可以补充但不能替代疑似MMP的临床病理评估。

英文摘要

Mucous membrane pemphigoid (MMP) is a chronic autoimmune subepithelial blistering disorder of mucosal surfaces that can cause irreversible scarring, blindness, or dysphagia. Early diagnosis is challenging because histopathology is nonspecific and conventional BP180 NC16A ELISAs have limited sensitivity. We evaluated ELISAs using full-length recombinant BP180 and a native BP180-α6β4 integrin complex in six patients with suspected oral or ocular MMP. The conventional NC16A ELISA was negative in four of six patients, and biopsy alone was often insufficient. Both assays provided useful serological support in a subset of cases when interpreted alongside direct immunofluorescence (DIF) and clinical phenotype, whereas in others DIF remained the primary basis for diagnosis. These assays can therefore complement, but not replace, clinicopathological assessment in suspected MMP.